Adenoid cystic carcinoma

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Adenoid cystic carcinoma
Diagnosis in short

Adenoid cystic carcinoma. H&E stain.

LM cribriform architecture (other patterns: solid, cords, (bilayered) tubules), cystic spaces filled with basophilic material, scant cytoplasm in most cells, nucleus - small, hyaline stroma
LM DDx pleomorphic adenoma, epithelial-myoepithelial carcinoma
Site salivary gland, breast, lung, skin, trachea, others

Signs mass
Prevalence relatively common malignant salivary gland tumour
Treatment surgical excision

Adenoid cystic carcinoma, abbreviated AdCC, is a malignant tumour commonly seen in the salivary gland.

General

  • Common malignant neoplasm of salivary gland.[1]
  • May occur in the skin.[2]
  • AKA cylindroma.[3]
  • Composed of ductal cells and myoepithelial cells; myoepithelial cells > ductal cells.

Microscopic

Features:

  • Cribriform architecture or pseudoglandular spaces (classic pattern) - important feature.
    • Other patterns: solid, cords, (bilayered) tubules.
    • Cystic spaces filled with basophilic material (that is PAS +ve) - key feature.
  • Scant cytoplasm in most cells (myoepithelial cells) - clear/eosinophilic.
    • Moderate eosinophilic cytoplasm in the (rare) ductal cells.
  • Nucleus - small.
    • May be angulated (carrot-shaped) - myoepithelial cells; round/ovoid in ductal cells.
  • Hyaline stroma.

Memory device:

  • AdCC - mostly DNA (scant cytoplasm), distinct nucleus (carrot-shaped).

Notes:

DDx:

Images

www

Grading

Based on solid component:

  • Low grade = tubules and cribriform structures only; no solid component.
  • Intermediate grade = solid component <30%.
  • High grade = solid component >=30%

Stains

Special stains:

  • PAS +ve material - cystic spaces.[4]

IHC

Features:[5]

  • CD117 +ve.
  • Cyclin D1 +ve/-ve.

Others:[6]

Note:

  • Myoepithelial markers (e.g. calponin, actin) +ve.
    • Typically -ve in PLGA.

Molecular

Features:[7]

  • t(6;9) MYB-NFIB.
    • Seen in ~50% of cases.
    • Worse prognosis if present, esp. if fusion assoc. with transcription.

See also

References

  1. Krüll, A.; Schwarz, R.; Engenhart, R.; Huber, P.; Lessel, A.; Koppe, H.; Favre, A.; Breteau, N. et al. (1996). "European results in neutron therapy of malignant salivary gland tumors.". Bull Cancer Radiother 83 Suppl: 125-9s. PMID 8949764.
  2. Wick, MR.; Swanson, PE. (Feb 1986). "Primary adenoid cystic carcinoma of the skin. A clinical, histological, and immunocytochemical comparison with adenoid cystic carcinoma of salivary glands and adenoid basal cell carcinoma.". Am J Dermatopathol 8 (1): 2-13. PMID 3010759.
  3. Chest. May 1957. Vol. 31. No. 5. PP. 493-511. http://www.chestjournal.org/content/31/5/493.abstract
  4. URL: http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970070-5. Accessed on: 12 May 2011.
  5. Sequeiros-Santiago, G.; García-Carracedo, D.; Fresno, MF.; Suarez, C.; Rodrigo, JP.; Gonzalez, MV. (May 2009). "Oncogene amplification pattern in adenoid cystic carcinoma of the salivary glands.". Oncol Rep 21 (5): 1215-22. PMID 19360297.
  6. Thompson, LD.; Penner, C.; Ho, NJ.; Foss, RD.; Miettinen, M.; Wieneke, JA.; Moskaluk, CA.; Stelow, EB. (Sep 2013). "Sinonasal Tract and Nasopharyngeal Adenoid Cystic Carcinoma: A Clinicopathologic and Immunophenotypic Study of 86 Cases.". Head Neck Pathol. doi:10.1007/s12105-013-0487-3. PMID 24037641.
  7. Mitani, Y.; Rao, PH.; Futreal, PA.; Roberts, D.; Stephens, P.; Zhao, YJ.; Zhang, L.; Mitani, M. et al. (Oct 2011). "Novel Chromosomal Rearrangements and breakpoints at the t(6;9) in Salivary Adenoid Cystic Carcinoma: association with MYB-NFIB chimeric fusion, MYB expression, and clinical outcome.". Clin Cancer Res. doi:10.1158/1078-0432.CCR-11-1870. PMID 21976542.